Spur cell anemia (acanthocytosis) complicating idiopathic hemochromatosis.

A 65-year-old male developed a rapidly progressive disease characterized by severe hemolysis, with spur cells (acanthocytes) and liver disease. Autopsy findings were consistent with a diagnosis of idiopathic hemochromatosis. Investigation of the patient's family uncovered four out of five first degree relatives with significantly raised serum ferritin levels. A sister had biopsy proven hemochromatosis. Spur cell anemia is a recognized, though rare, complication of alcoholic liver disease and indeed the patient had a regular alcohol intake of up to 50 g daily. Although the alcohol intake could have contributed to the formation of spur cells, the possible association with hemochromatosis should be considered. A diagnosis of hemochromatosis has important implications for family members.